Study the severity and outcome of children with interstitial lung disease

The child interstitial lung diseases (chILD) are a group of chronic lung diseases resulting from pathological changes of alveolar wall and capillary units.The epidemiology of the various forms of chILD is difficult to establish. small studies have suggested an approximate incidence of 0.5-0.8 cases per 100 000 children The prevalence is estimated to be 3.6 and 1.32 per million in the UK and Germany, respectively. but there are no recorded studies for its prevalence in Egypt due to the lack of standardized definitions , the inadequacy of organized reporting systems, and the variety of pathological conditions. In addition, clinical presentation is often nonspecific, contributing to a poor recognition of these disorders and confusion with other chronic pulmonary diseases .

childhood interstitial lung disease may be considered if the child meets at least three of the criteria presented (1) Respiratory symptoms (e.g., cough, difficult breathing, exercise intolerance). (2). Respiratory signs (e.g., retractions, tachypnea, clubbing, failure to thrive). (3). Hypoxemia. (4). Diffuse parenchymal abnormalities on chest imaging .

The causes of chILD often remain undetermined. These diffuse lung disorders are chronic, and often have high morbidity and mortality .

Mortality from childhood interstitial lung diseases ranges from 15 to 60% in different research reaching up to 100% in genetic and developmental causes.

There are many challenges and uncertainties for the early and correct diagnosis of interstitial lung disease in the pediatric patient. Three major challenges include, First , the fact that interstitial lung disease is less common in infants and children compared with adults. Therefore, most clinicians and radiologists are less familiar with considering and recognizing interstitial lung disease in this patient population.

Second, the clinical manifestations of interstitial lung disease particularly in infants and the young child are often subtle, highly variable, and typically nonspecific, such as dyspnea, tachypnea, crackles, and hypoxemia.

Lastly, there are currently no pathognomonic clinical or laboratory criteria for the diagnosis of interstitial lung disease in pediatric patients.

Some of the major uncertainties involving interstitial lung disease in this population include the decrease of information regarding the natural history of interstitial lung disease in childhood,the lack of understanding of the role of specific host factors in the pathogenesis of interstitial lung disease, and the absence of information on prognostic indicators in interstitial lung disease in childhood and more particularly in infants. Due to the above-stated challenges and uncertainties, evaluation and diagnosis of interstitial lung disease in childhood and particularly in infants has been markedly limited in the past.

Interstitial lung diseases may be classified a specific, suspicious, or non -specific diagnosis . A specific diagnosis was assigned to those cases in which a diagnosis was made with confidence based on careful consideration of clinical presentation , laboratory evaluations, and invasive diagnostic studies , such as lung biopsy.

A suspicious diagnosis was assigned to those cases in which the clinical presentation suggested a disorder (such as hypersensitivity pneumonitis from bird exposure) that could not be confirmed conclusively by diagnostic evaluation.

Non - specific diagnosis was assigned to those cases in which no specific or suspicious diagnosis could be made despite complete diagnostic evaluation.

The appropriate and definitive investigation are lung biopsy in order to establish a precise diagnosis so At our hospital, we face many obstacles regarding the diagnosis .

The present study will be on suspicious and specific groups

Inclusion Criteria: All children more than 1 month and less than 18 years of age.

Suspected cases with:

1. tachypnea , Hypoxemia less than 90% in room air , rales and wheezes in absence of chest infection , retraction or deformed chest wall .
2. pulmonary hypertension by ECG or echocardiography.
3. x-ray and CT chest infiltrates.
4. Duration of illness more than 1 month.

Specific cases with:

1. symptoms and signs of suspected cases.

2. Lung biopsy.

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   Exclusion Criteria:• All children less than 1 month and more than 18 years

   • congenital heart disease.
   • bronchopulmonary dysplasia.
   • Telangiectasia.
   • Duration of illness less than 1 month

All children with suspected ILD that present to Assiut University Hospital Units